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N714K

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
AsparagineLysine at position 714 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Asparagine → Lysine at position 714 in wolframin's C-terminal lumenal domain. ClinVar Conflicting classifications. AlphaMissense 0.992 (near-maximum), DynaMut2 ΔΔG -0.44 kcal/mol (destabilising). The FOURTH Atlas variant at position 714 (with N714T, N714S, and D771H in the same network).

Interactive 3D Structure

Wild-type reference
Wild-type N714 — hydrogen bond to I767
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DynaMut2 mutant · N714K
Mutant K714 — hydrogen bond to A716 lost (7 contacts lost)
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Bond changes · DynaMut2 interaction analysis

7 lost5 gained6 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondI712Gained
Hydrogen bondA716Lost
Hydrogen bondE717E717Preserved
Hydrogen bondS718S718Preserved
Hydrogen bondI767Lost
Hydrogen bondD771Lost
Polar contactI712Gained
Polar contactA716Lost
Polar contactE717E717Preserved
Polar contactS718S718Preserved
Polar contactI767Lost
Polar contactD771D771Preserved
Van der WaalsI712Gained
Van der WaalsA716Lost
Van der WaalsE717Lost
Van der WaalsS718Gained
HydrophobicI712Gained
HydrophobicE717E717Preserved

Lost / gained / preserved interatomic contacts at the variant residue, from the DynaMut2 (Arpeggio) interaction analysis of the wild-type and energy-minimized mutant structures.

Computational Predictions

DynaMut2 ΔΔG
-0.44kcal/mol
Destabilising — mild
AlphaMissense
0.992
LPath
AlphaFold pLDDT
87
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditions(no specific conditions catalogued — conflicting classifications)
InheritanceConflicting classifications.
Population frequency (gnomAD v4)Absent from gnomAD v4
cDNA changec.2142C>G
ClinVar accessionVCV002831353
Last evaluated2025/02/16 00:00

Not observed in ~730k individuals — consistent with a rare allele (ACMG PM2_supporting).

Structural Context

Position 714 same neighbor environment as N714T/N714S: SER715 (2.4 Å), ASP713 (2.5 Å), PHE770 (4.4 Å), ALA716 (4.4 Å), ASP771 (4.7 Å).

Replacing N714 with lysine introduces a positive charge into the D713-D771 polar network — adjacent to two existing negative charges. The K714 amine likely forms salt bridges with D713 and/or D771, restructuring the polar network entirely (where the wild-type N714 amide H-bonded but did not carry charge).

The |ΔΔG| of 0.44 reflects fold accommodation. AlphaMissense's 0.992 (near-maximum) confirms severe functional consequence — the charge introduction disrupts the wild-type partner-recognition geometry.

Amino-acid chemistry
Asparagine (N) → Lysine (K) — polar amide replaced by large positively-charged amine. H-bonding capacity preserved but charge introduced.
Position in the protein
C-terminal lumenal domain · position 714 in the ER lumen (pLDDT 87). Same position as N714T and N714S.

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.44 — fold survives. AlphaMissense 0.992 (near-maximum) confirms severe functional consequence.

Mechanism is charge introduction into the D713-D771 polar network. Therapeutic strategy: same microregion as N714T, N714S, D771H.

Why this matters

N714K is the FOURTH Atlas variant at position 714 (N714T, N714S, N714K) plus D771H. The D713-N714-D771 polar network is the densest multi-variant target in the lumenal domain — at least four convergent rescue opportunities.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the N714K PDF below and upload it to Wolfram Intelligence to generate therapeutic-strategy proposals — guanidinium mimetics, sigma-1 agonist docking, NAC thiol-capping. NAC is already on the bench-testing list.

Download N714K PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal